Within a five-generation Michigan-Kentucky pedigree, originating from a proband with pancreatic agenesis and homozygous for the PDX1 mutation Pro63fsx60, both the proband’s parents exhibited fasting hyperglycemia (155 and 147 mg/dL, respectively), elevated post-glucose plasma glucose levels (265 and 160 mg/dL, respectively), significantly increased fasting insulin (98 and 39 μU/mL, respectively) and C-peptide (8.0 and 5.0 ng/mL, respectively) levels, along with elevated postprandial levels (insulin 284 and 100 μU/mL, C-peptide 21.4 and 13.7 ng/mL, respectively) (40). The gene discussed is INS; the disease is Hyperglycemia.